title: Management of a patient with familial idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF), the most common subtype of idiopathic interstitial pneumonia, is a chronic progressive lung disease with a very high mortality. Usually diagnosis is established in adults older than 50 years and most cases are considered to be sporadic. Individuals with a familial form of IPF have at least one affected member in the same primary biological family and account for less than 5% of total patients. Sporadic and familial IPF are clinically and histologically indistinguishable from one another, although some familial forms appear to develop at an earlier age and exhibit different patterns of gene transcription. This case study describes the early clinical course of a patient diagnosed with a familial form of IPF.
authors.affiliation: Institute of Pneumology at the University Witten/Herdecke, Clinic for Pneumology and Allergology, Centre of Sleep Medicine and Respiratory Care, Bethanien Hospital Solingen, Germany. sebastian.boeing@klinik-bethanien.de
authors.forename: S
authors.initials: S
authors.lastname: Böing
authors.forename: S
authors.initials: S
authors.lastname: George
authors.forename: L
authors.initials: L
authors.lastname: Hagmeyer
mesh_terms: D002908:Chronic Disease; D006801:Humans; D054988:Idiopathic Interstitial Pneumonias; D054990:Idiopathic Pulmonary Fibrosis; D011658:Pulmonary Fibrosis / Q000175:diagnosis